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This 5-year-old boy has 20/20 vision in each eye and his refraction is
OD and OS +.50 sphere. Head posture is as shown below with head
tilt to the left.
Adduction is limited in both eyes, but enophthalmos and up and down
shoot are seen mostly in the right eye. When assuming the head
posture shown, this boy fuses the Titmus stereo fly (4,000 seconds).
How would you classify this Duane syndrome?
What treatment would you recommend?
Expert Commentary
Comments of Arthur L. Rosenbaum, M.D.
This child appears to have asymmetric
bilateral exotropic Duane syndrome. The
pictures clearly substantiate this diagnosis in
the right eye and to a lesser extent in the
left eye. I do not actually see globe retraction
or narrowing of the palpebral fissure in the
left eye on attempted adduction. However,
there is an unequivocal inability to fully
adduct the left globe. I have never seen a
compensatory head posture using a large head tilt, as shown in the
photos. I assume that he is adopting this posture to somehow neutralize
the upshoot and/or downshoot of the right globe in adduction. At
any rate, he seems to be a fusing patient with an anomalous head
posture and therefore a surgical candidate.
I would recommend a right lateral rectus Y-splitting procedure with
larger recession of each half of the split right lateral rectus and
transposition of the superior half superiorly and the inferior half
inferiorly.
This should reduce the exotropia in the primary position and improve
the upshoot and downshoot as well as his head posture.
I would not recommend treatment of the left eye at this time.
In such patients, we are beginning to perform disinsertion of the right
lateral rectus muscle with attachment to the lateral orbital wall
accompanied by a partial transposition of the lateral halves of the
superior and inferior rectus muscles to the lateral rectus insertional
stump, with posterior fixation. The first few cases that we have performed
have done extremely well, although some have required subsequent
medial rectus recession, since the patient no longer has any lateral
rectus function. However, I think it is too early to recommend this
type of treatment at the present time. The time honored option is to
weaken the right lateral rectus muscle, as described in the first part of
this discussion.